Oxford cases in medicine and surgery new medical books

For example, acute coronary syndrome is a common cause of chest pain, viral costochondritis is not.. List of abbreviations xxiHiB Haemophilus infl uenzae B HIT heparin-induced thrombocy

Oxford Cases in Medicine and Surgery

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British Library Cataloguing in Publication Data

in this work

Th ere is an abundance of excellent medical and surgical textbooks, written in both traditional and more novel formats However, in a climate in which the content and mode of delivery of medical education remain in constant fl ux there remains

a need for new resources that stimulate interest in the reader as well as providing

the important and relevant facts Oxford Cases in Medicine and Surgery fulfi ls this

need Th is book’s uniqueness – and its educational value – stems from the way that the authors have approached the learning aspect from direct clinical symptoms, highlighting the most important diff erential diagnoses but also explaining how to diff erentiate them Th is approach represents the book’s real strength, mirroring

as it does the integrated systems-based approach that is commonly used by many medical schools

In my experience as a clinical teacher, course organiser, and examiner over the past decade, this is the fi rst book that has attempted to bring together, and explain from a basic science concept, the reasons for the clinical picture or condition Th is will help readers enormously, whether they are under-graduate or post-graduate medical, dental, or nursing students It is an important book for those who wish to understand the reasons for clinical presentations and their diff ering management

Mr Christopher LH Chan Senior Lecturer/Honorary Consultant Surgeon Barts and Th e London School of Medicine and Dentistry

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Why we wrote this book

Th e inspiration for this book comes from our time as medical students Th e problem we found with existing textbooks was twofold

Firstly, most books are organized by pathology For example, they may have ters on ‘cardiology’ that then discuss specifi c conditions, like ‘myocardial infarction’,

chap-in detail But patients do not present with ready-made diagnoses like ‘myocardial infarction’ Th ey present with symptoms, such as ‘chest pain’, which could be a myo- cardial infarction – but could also be anything from refl ux oesophagitis to aortic dissection

Secondly, there are also textbooks based around cases rather than pathologies Our experience is that these tend to skip over the diagnostic approach too quickly,

in order to move on to a discussion of the underlying disease Many give the reader

so much information in the initial case presentation that the diagnosis is virtually

made for you For example, a ‘62-year-old diabetic male with sudden onset, ing chest pain; tachycardia on examination; ST elevation on his ECG, plus raised troponins’ has a myocardial infarction But by giving so much information upfront,

crush-these books neglect to address what many students fi nd most challenging – how

do you decide what information to collect in order to make a diagnosis? Patients present with symptoms such as ‘chest pain’ and it is your job to elicit the key clues

on history and examination, and to arrange the key investigations that will confi rm that this is a myocardial infarction and rule out other diagnoses.

Knowing what to do when faced simply with ‘confusion’ or ‘abdominal pain’ can be daunting and tricky – we know, and that is what motivated us to write this book.

We hope this book will help you start thinking like a diagnostician from your fi rst day on the wards Th us, we hope you will be able to work out why your patient is short of breath or has abdominal pain in a way that is safe and effi cient, and avoids you missing important diagnoses Even with detailed knowledge of anatomy, physi- ology, biochemistry, pathology, history-taking, examination skills, and data inter- pretation, it can be diffi cult to integrate everything when faced with acutely ill patients on the wards We benefi ted greatly from case-based seminars that taught

us a hypothesis-driven, logical, step-by-step approach to diagnosis Our hope is that this book emulates the teaching that we found so benefi cial

Finally, we wanted to write a workbook that students will enjoy using and where even the simplest concepts are clearly explained

The need for a logical diagnostic approach

Looks like an elephant Sounds like an elephant Smells like an elephant ably an elephant Experienced clinicians often use pattern recognition to guide

Prob-diagnosis As a student, you will begin to do this rapidly for conditions that you will encounter frequently – chances are that, by now, you easily recognized that the 62-year-old diabetic male mentioned above was having a myocardial infarction

of But misdiagnosis due to (incorrect) pattern recognition can have disastrous sequences – you could inadvertently thrombolyse a patient you thought was having

con-a myoccon-ardicon-al infcon-arction but con-actucon-ally hcon-ad con-an con-aortic dissection Th is is one reason why

it is important to always follow a logical diagnostic approach.

Looks like an elephant Sounds like a lion Not sure what it smells like Must be

a ? You cannot recognize a pattern you have never seen before, an especially big

problem for the inexperienced medical student starting their clinical placements

On other occasions, the symptoms may not fi t any known pattern, and even enced clinicians may struggle initially with the diagnosis Th is is another reason for having a logical diagnostic approach.

experi-A logical approach to diagnosis

Below is an outline of the diagnostic strategy we have used throughout this book We recognize that, over time, everyone develops their own diagnostic strategy and that tutors may teach you diff ering approaches Th is is simply one that has worked for us.

‘50-year-old male with chest pain’ It is tempting to assume that he is having a

myo-cardial infarction, like the 62-year-old diabetic male mentioned above However,

Step 1

• : Th ink of all the things that could cause this presentation Use anatomy,

a surgical sieve (e.g INVITED MD), etc to come up with as long a list as possible.

Step 2

• : Highlight from your list the most common causes For example, acute

coronary syndrome is a common cause of chest pain, viral costochondritis is

not Mark the ones that you must exclude because they are lethal In the case

of chest pain, Boerhaave’s perforation of the oesophagus is important as, if untreated, it carries a 100% mortality.

Step 3

• : Th ink of key clues in the patient history for each of the diagnoses For example, patients with Boerhaave’s perforation of the oesophagus invariably give a history of vomiting immediately before onset of the pain Now take a history that deliberately tries to pick up these clues, rather than just going through a set of ‘standard’ questions which may miss things Also consider the patient themselves (e.g their age, occupation, etc.) and how this aff ects the

relative likelihoods of your diff erential diagnoses Has the patient’s history or epidemiological factors made any diagnoses more/less likely?

Step 4

• : Th ink of key clues on examination for your diagnoses For example, patients with a pneumothorax will have an area of the chest that is hyperex- panded, hyper-resonant to percussion, with absent breath sounds Perform a

thorough examination looking for these clues Have your examination fi ings made any diagnoses more/less likely?

Step 2: What is most likely?

What must I exclude (*)?

Step 3: Key clues on history?

Step 4: Key clues on examination?

Step 5: Key clues on basic investigations?

Step 6: Patient improving with management?

Pulmonary embolism*

Pleurisy

Anxiety

MyopericarditisAortic aneurysmCoronary spasmCholecystitisPancreatitis

Acute coronary syndrome*

Pulmonary embolism*

PleurisyAnxietyMyopericarditisAortic aneurysmCoronary spasmCholecystitisPancreatitis

Acute coronary syndrome*

Pneumothorax*

Aortic dissection*

Boerhaave’s perforation*

Peptic ulcer diseaseStable angina*

MusculoskeletalOesophagitisOesophageal spasmPulmonary embolism*

PleurisyAnxietyMyopericarditisAortic aneurysmCoronary spasmCholecystitisPancreatitis

Acute coronary syndrome*

PleurisyAnxietyMyopericarditisAortic aneurysmCoronary spasmCholecystitisPancreatitis

Acute coronary syndrome*

PleurisyAnxietyMyopericarditisAortic aneurysmCoronary spasmCholecystitisPancreatitis

50-year-old male with

Smoker with known COPD No risk factors for venous thrombosis

Upper left zone of chest is hyper- resonant with reduced air entry and reduced vocal resonance Chest not tender to palpation No signs of DVT in calves

Chest radiograph shows air in pleural space on left, with lung collapsing away from the upper left apex

Patient improves after insertion of a chest drain for pneumothorax Chest pain resolves completely

x Introduction

Step 5

• : Don’t order a set of ‘standard’ investigations Th ink about those tigations that will help confi rm or dismiss each diagnosis Also include those that are relevant for management Th us urea and electrolytes are necessary if a patient is put nil by mouth and on intravenous fl uids, or started on drugs that are renally excreted or potentially nephrotoxic Try to prioritize investigations into those that are more readily available (e.g an MRI head scan is not a viable option for everyone who presents with a fall) Also think about which investi- gations are safe for this patient – is radiation exposure necessary, is the woman

inves-pregnant, do they have contraindications to MRI? Th en ask yourself, have your investigation results made any diagnoses more/less likely?

Step 6

• : Always try to confi rm your diagnosis Is the patient getting better with

your management for your proposed diagnosis? If not, why not?

What this book is about

✓Common acute presentations: We cover 29 of the most common patient

presentations in acute general medicine (internal medicine for our American readers) and general surgery Th ese refl ect both the general medical and surgi- cal syllabus at UK medical schools and those presentations that you are most likely to encounter during clinical attachments

✓Diagnostic strategy: Th is book is primarily a diagnostic manual It should

equip the student with a framework for thinking about the most common general medical and surgical presentations

✓Pattern recognition: Th e cases are loosely based on real clinical scenarios,

although any likeness to a particular patient or individual is unintended Some cases represent stereotypical presentations of diseases, from which the stu- dent may begin to pick up pattern recognition skills Others illustrate more unusual presentations, and are designed to keep readers on their toes and remind them to keep an open mind at all times

✓Basic management: For completeness, we include a discussion of the basic

management for many of the diseases featured in the cases Points on agement cover the core knowledge expected of a medical student but are nec- essarily brief We have tried to highlight areas of contention, and to refer to landmark trials and guidelines where relevant Some of this is covered under our ‘viva questions’.

man-What this book is not about

✗Every possible diagnosis: It is not logistically possible to condense the

entirety of the medical and surgical syllabus into a book of this style – indeed such an attempt would run counter to the aims of this book Our aim is to

cover the most common presentations, and in so doing we also cover the most

common diagnoses We are fully aware that many diagnoses are not covered But our hope is that we have provided a framework that will enable the reader

to exclude the more common conditions, and be able to deal intelligently with clinical conundrums Th e reader should be equipped to recognize the salient features of the case in question, and know when to ask for specialist help

Introduction xi

A case-based book which attempted to cover all possible diagnoses which may

be encountered would not only be so long as to be unwieldy, but would also

run the risk of suggesting that pattern recognition is a surrogate for a rational

diagnostic strategy.

✗Basic sciences and clinical skills: Th is book does not aim to teach disease

pathology or how to take a history, examine a patient, and how to interpret

basic investigations (biochemistry, haematology, radiology) However, we

believe that this book can be fruitfully read alongside books and teaching

about basic science and clinical skills.

✗Specialities: It should also be noted that the cases covered refl ect only a

selection, albeit a broad one, of the diseases and presentations that a medical

student needs to cover Th e bulk of the omissions relate to the specialities (e.g

obstetrics and gynaecology, paediatrics, ear, nose, and throat, ophthalmology,

etc.) and general practice (family medicine)

✗Epidemiology: Th is book does not contain detailed epidemiological data

on the exact likelihood of diagnoses, because such data are rarely available

and hardly memorable Instead, we consider diagnoses to be either ‘common’,

‘occasional’, or ‘very rare’ Th is is based on data, where available, or the

cumula-tive experience of our senior reviewers (all of them consultants of many years’

standing)

✗Detailed management: Th is book does not focus on drug doses, surgical

techniques, or other details of management, because these can already be

found in other textbooks.

How to use this book

A workbook, not a reference text: Th is is intended to be an exercise text

where you ‘learn by doing’ Try to cover up the answers and work through the

text (without cheating!) to get the most out of it.

Find a presentation: We have structured this book by presenting

com-plaint, rather than pathology, because patients present with ‘chest pain’

rather than ‘aortic dissection’ For ease of reference there is also an index

by disease Each chapter can be read individually, so the student can read

those that relate to the presentation they last encountered or that was most

recently discussed Every chapter contains a core case, short cases, and viva

questions, in that order.

Core case: Each core case is a clinical problem that walks you through the

diagnostic approach Th e information the clerking doctor might receive is

provided in an initial box, followed by a question Th e answer follows, with

another section with clinical information and another question, and so on

Short cases: Th e short case ‘vignettes’ are designed to highlight some of the

other conditions that can present in a similar manner (indeed, with the same

symptom) Th ey will help develop your ‘pattern recognition’ of some diseases,

but also remind you that pathologies can masquerade as one another, hence

the need for a logical approach

Viva questions: Th ese questions are designed to test aspects of anatomy,

pharmacology, physiology, etc related to the cases We hope they will prepare

xii Introduction

the reader for the inevitable quizzing that occurs on teaching ward rounds or

in theatres/operating rooms.

Graphical features: Questions are on a red background Font sizes in a diff

er-ential diagnosis illustrate how likely a diagnosis is (or isn’t) Important points are in red or bold text.

Miss P J Clarke, Dr J Dwight, Mr A Handa, Dr T Lancaster, and Dr T Littlewood: thank you for sharing your invaluable clinical and educational experience with us, and for tirelessly reviewing all of the chapters over the past 2 years.

Dr P Dennis and Dr T Lancaster: thank you for your case-based seminars, which inspired this book We hope to have captured the essence of what you taught us as medical students.

Dr C Conlon, Professor T Hope, Dr N Meston, Mr R Mihai, Dr A Slater, sor C Tapper, Dr W Th evathasan, Dr C M Norris, and Dr T Walker: thank you for specialist advice when we were out of our depth.

Profes-Dr R Graham and Profes-Dr J Teh: thank you for helping us obtain elusive images Miss C Connelly, Miss H Edmundson, and the staff from OUP: thank you for believing in our project, encouraging us, and mentoring us as fi rst time authors Emily: thank you for being so patient and discreet

Rachel: thank you for cooking your boys endless amounts of brain food.

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Dr J Dwight MD FRCP

Consultant Cardiologist, Department

of Cardiology, John Radcliff e Hospital,

Oxford

Dr T Lancaster MRCP MRCGP

General Practitioner and Director

of Clinical Studies, John Radcliff e

Hospital, Oxford

Dr T Littlewood MD FRCP FRCPath

Consultant Haematologist,

Department of Haematology, John

Radcliff e Hospital, Oxford

Mr A Handa MBBS FRCS

Clinicial Tutor & Consultant Vascular Surgeon, Nuffi eld Dept of Surgery, John Radcliff e Hospital, Oxford

Miss P J Clarke MD FRCS

Consultant Breast & General Surgeon, John Radcliff e Hospital, Oxford Editorial advisors

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15 Right upper quadrant (RUQ) pain 291

16 Right iliac fossa (RIF) pain 307

17 Left iliac fossa (LIF) pain 323

26-29 22-25

12-14

15-18

19-21 7-11 1-3

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List of abbreviations

AAA abdominal aortic aneurysm

ABC airways, breathing, and circulation

ABG arterial blood gas

ABPI ankle–brachial pressure index

ACA anterior cerebral artery

ACE angiotensin-converting enzyme

ACEi angiotensin-converting enzyme inhibitor

ACTH adrenocorticotropic hormone

ADH antidiuretic hormone

ADP adenosine diphosphate

A&E Accident and Emergency [Department]

AFP alpha-fetoprotein

ALP alkaline phosphatase

ALT alanine aminotransferase

AMA antimitochondrial antibodies

AMTS Abbreviated Mental Test Score

ANA antinuclear antibodies

APTT activated partial thromboplastin time

ARBs angiotensin II receptor blockers

ARDS acute respiratory distress syndrome

ASIS anterior superior iliac spine

ASMA antismooth muscle antibodies

AST aspartate aminotransferase

ATLS advanced trauma life support

AV [node] atrioventricular [node]

BCG bacille Calmette–Guérin [vaccine against tuberculosis]

b.d twice a day [drug dosing]

BMI body mass index

BNF British National Formulary

BP blood pressure

BPH benign prostatic hyperplasia

bpm beats per minute

BPPV benign paroxysmal positional vertigo

BTS British Th oracic Society

CABG coronary artery bypass graft

cANCA cytoplasmic-staining antineutrophil cytoplasmic antibodies

CBT cognitive behavioural therapy

CCB calcium-channel blocker

CCK cholecystokinin

CCP cyclic citrullinated peptide

CEA carcinoembryonic antigen

CLO columnar lined oesophagus

CMV cytomegalovirus

xx List of abbreviations

CNS central nervous system

COCP combined oral contraceptive pill

COPD chronic obstructive pulmonary disease

COX cyclooxygenase

CRP C-reactive protein

CSF cerebrospinal fl uid

CT computed tomography

CT-KUB CT of kidneys, ureters, and bladder

CTPA CT pulmonary angiogram

CVP central venous pressure

DCBE double-contrast barium enema

DIC disseminated intravascular coagulation

DSM-IV Diagnostic and Statistical Manual of Mental Disorders, 4th

edition

DVLA Driver and Vehicle Licensing Agency

DVT deep vein thrombosis

EBV Epstein–Barr virus

ECG electrocardiogram

EEG electroencephalogram

eGFR estimated glomerular fi ltration rate

ELISA enzyme linked immunosorbent assay

ENT ear, nose, and throat

ERCP endoscopic retrograde cholangiopancreatography

ESR erythrocyte sedimentation rate

ESWL extracorporeal shock wave lithotripsy

EUS endoscopic ultrasound

FAP familial adenomatous polyposis

FATP1 fatty acid transporter protein 1

FBC full blood count

FER forced expiratory ratio

FEV1 forced expiratory volume in 1 second

FiO2 fraction of inspired oxygen

FMTC familial medullary thyroid carcinoma

FNA fi ne needle aspiration

FOBT faecal occult blood test

FVC forced vital capacity

G6PDH glucose-6-phosphate dehydrogenase

GCA giant cell arteritis

GCS Glasgow Coma Scale

β-HCG β-human chorionic gonadotropin

HDU high-dependency unit

List of abbreviations xxi

HiB Haemophilus infl uenzae B

HIT heparin-induced thrombocytopenia

HNPCC hereditary non-polyposis colorectal cancer

HOCM hypertrophic obstructive cardiomyopathy

HONK hyperosmotic non-ketotic [coma/acidosis]

HPOA hypertrophic pulmonary osteoarthropathy

HR heart rate

HRT hormone replacement therapy

IBD infl ammatory bowel disease

IBS irritable bowel syndrome

ICD-10 WHO International Statistical Classifi cation of Diseases and

Related Health Problems, 10th revision

ICDs implantable cardioversion devices

Ig immunoglobulin

IM intramuscular

INO internuclear ophthalmoplegia

INR international normalized ratio

IPAA ileal pouch–anal anastomosis

ITP immune thrombocytopenic purpura

ITU intensive therapy unit

IV intravenous

IVC inferior vena cava

IVF in vitro fertilization

IVP intravenous pyelogram/pyelography

IVU intravenous urogram/urography

JVP jugular venous pressure

LABA long-acting β2-agonist

LACA long-acting anticholinergic

LAD left anterior descending coronary artery

LDH lactate dehydrogenase

LDL low-density lipoprotein

LFTs liver function tests

LHRH luteinizing hormone-releasing hormone

LIF left iliac fossa

LMN lower motor neuron

LMWH low-molecular weight heparin

LNG-IUD levonorgestrel-releasing intrauterine device

LP lumbar puncture

LPL lipoprotein lipase

LUQ left upper quadrant

LUTS lower urinary tract symptoms

MCA middle cerebral artery

MC&S microscopy, culture, and sensitivities

xxii List of abbreviations

MRCP magnetic resonance cholangiopancreatography

MRI magnetic resonance imaging

MRU magnetic resonance urogram

MSU mid-stream urine

MTC medullary thyroid carcinoma

NBM nil by mouth

NG nasogastric [tube]

NICE National Institute for Health and Clinical Excellence

NIV non-invasive ventilation

NSAID non-steroidal anti-infl ammatory drug

NSCLC non-small cell lung cancer

NSTEMI non-ST elevation myocardial infarction

NUD non-ulcer dyspepsia

NYHA New York Heart Association

o.d once a day [drug dosing]

OGD oesophagogastroduodenoscopy

OSCE Objective Structured Clinical Examination

PaCO2 arterial partial pressure of carbon dioxide

PaO2 arterial partial pressure of oxygen

pANCA perinuclear-staining antineutrophil cytoplasmic autoantibodies

PBC primary biliary cirrhosis

PCA posterior cerebral artery

PCD primary ciliary dyskinesia

PCNL percutaneous nephrolithotomy

PCOM posterior communicating artery

PE pulmonary embolism

PEF peak expiratory fl ow

PEFR peak expiratory fl ow rate

PET positron emission tomography

Pi inorganic phosphate

PK pyruvate kinase

PMN polymorphonuclear leucocyte

PPAR peroxisome proliferator-activated receptor

PPI proton-pump inhibitor

PSA prostate-specifi c antigen

PSC primary sclerosing cholangitis

PT prothrombin time

PTH parathyroid hormone

PTHrP parathyroid hormone-related peptide

PUJ pelvi-ureteric junction

q.d.s four times a day [drug dosing]

RAPD relative aff erent papillary defect

RBC red blood cell [count]

RCA right coronary artery

RIF right iliac fossa

RLN recurrent laryngeal nerve

RUQ right upper quadrant

SACD subacute combined degeneration of the cord

SAH subarachnoid haemorrhage

List of abbreviations xxiii

SALT speech and language therapist

SC subcutaneous

SCLC small cell lung cancer

SIADH syndrome of inappropriate ADH secretion

SLE systemic lupus erythematosus

SOL space-occupying lesion

SSRV small structured round virus

STEMI ST elevation myocardial infarct

SVC superior vena cava

T3 tri-iodothyronine

TB tuberculosis

t.d.s three times a day [drug dosing]

TFTs thyroid function tests

TG thyroglobulin

TIA transient ischaemic attack

TIBC total iron-binding capacity

TIPS/TIPSS transjugular intrahepatic portosystemic shunt

TMJ temporomandibular joint

TNF tumour necrosis factor

tPA tissue plasminogen activator

TPN total parenteral nutrition

TRAM tranverse rectus abdominis myocutaneous [fl ap]

TSH thyroid-stimulating hormone

TTG tissue transglutaminase

TURP transurethral resection of the prostate

TWOC trial without catheter

UC ulcerative colitis

U&Es urea and electrolytes

UMN upper motor neuron

UTI urinary tract infection

VTE venous thromboembolism

WCC white cell count

WLE wide local excision

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Headache

2 Headache

1

Core cases

Read through the sections in red, covering up the sections in white that follow

so that you don’t see the answer At the end of each red section, try to answer the question before reading on.

Th e sinister causes can be remembered using the mnemonic VIVID :

V ascular: subarachnoid haemorrhage (SAH), haematoma (subdural or dural), cerebral venous sinus thrombosis, cerebellar infarct

extra-I nfection: meningitis, encephalitis

V ision-threatening: temporal arteritis†, acute glaucoma, pituitary apoplexy, posterior leucoencephalopathy, cavernous sinus thrombosis

I ntracranial pressure (raised): space-occupying lesion (SOL; e.g tumour,

abscess, cyst), cerebral oedema (e.g trauma, altitude), hydrocephalus, nant hypertension

D issection: carotid dissection

† Note that temporal arteritis is another name for giant cell arteritis, a systemic vasculitis Th e term temporal arteritis is more common when headache is the presenting symptom

Th e approach to headache is the same as that to pain anywhere in the body: you need to start by characterizing the pain One useful way of doing this is by following another mnemonic, SOCRATES :

S ite of pain, and has it moved since it began?

O nset of pain – was it sudden or gradual, and did something trigger it?

C haracter of pain – stabbing, dull, deep, superfi cial, gripping, tearing, burning?

R adiation of pain – has the pain spread?

What sinister causes must you rule out?

Taking a good history is key to any diagnosis, but particularly so when tackling headache as the symptom is so subjective and examination fi ndings are often unhelpful

With a mental list of the sinister causes, what questions will you ask fi rst in the history? What ‘red fl ags’ will help you exclude the sinister causes?

Core cases 3

1

A ttenuating factors – does anything make the pain better (position? medications?)

T iming of pain – how long has it gone on for, has it been constant or coming and

going?

E xacerbating factors – does anything make the pain worse (moving? breathing?)

S everity – on a scale of 0 to 10, where 10 is the worst pain ever (e.g childbirth).

In addition, you should enquire about the presence or absence of the following ‘red

fl ags’:

medical presentation Combined with headache, SAH needs exclusion If

there is a history of head injury, it could suggest a subdural haematoma

(fluctuating consciousness) or extradural haematoma (altered

consciousness following a lucid interval) Meningitis and encephalitis can

also affect consciousness.

• Sudden onset, worst headache ever Suggests SAH, with blood in the

cer-ebrospinal fl uid (CSF) irritating the meninges It can be informative to ask the

patient whether they remember the exact moment when the headache started

– a very severe headache of almost instantaneous onset is characteristic of

SAH Patients describe it like, for example, ‘being hit on the head with a

base-ball bat’.

• Seizure(s) or focal neurological defi cit (e.g limb weakness, speech diffi

cul-ties) Suggests intracranial pathology.

• Absence of previous episodes Recurrent episodes are usually less sinister

A new onset of headache suggests a new pathology In someone over 50 years

old, a new onset headache should raise your suspicions of temporal arteritis

until proven otherwise.

• Reduced visual acuity Temporal arteritis is common in older patients

Tran-sient blindness (amaurosis fugax) is usually due to a tranTran-sient ischaemic attack

(TIA), but these rarely produce a headache In the context of headaches, loss

of vision can be due to temporal arteritis, carotid artery dissection causing

decreased blood fl ow to the retina, or acute glaucoma.

• Persistent headache, worse when lying down, and coupled with early

morn-ing nausea Suggests raised intracranial pressure Th is is worse when lying

fl at for prolonged times (e.g overnight) due to the eff ect of gravity, but can

even occur when the patient is bending over Headaches that are worse when

standing up suggested reduced intracranial pressure and are common after a

lumbar puncture (LP), but these are not sinister and resolve with hydration

and lying down for several hours.

• Progressive, persistent headache Th is could be an expanding SOL (e.g

tumour, abscess, cyst, haematoma).

• Constitutional symptoms Weight loss, night sweats, and/or fever may

sug-gest malignancy, chronic infection (e.g tuberculosis), or chronic infl

amma-tion (e.g temporal arteritis).

4 Headache

1

Basic observations

score, although it is likely to already be obvious from the history taking Th e signifi cance of altered consciousness is discussed above.

• Blood pressure and pulse Check for malignant hypertension

• Temperature Fever and headache suggests meningitis or encephalitis.

Focal neurological signs

Note that the list below is not exhaustive.

• Focal limb defi cit Makes intracranial pathology more likely.

• Th ird nerve palsy Th is consists of ptosis (droopy eyelid), mydriasis (dilated pupil), and an eye that is deviated down and out One cause is an SAH due to

a ruptured aneurysm of the posterior communicating artery (PCOM) PCOM aneurysms are a cause of headache.

• Sixth nerve palsy Convergent squint and/or failure to abduct the eye

later-ally Th is nerve can be compressed either directly by a mass or indirectly by raised intracranial pressure Remember that the sixth nerve has the longest intracranial course and is therefore most likely to get compressed at some point.

• Twelfth nerve palsy Look for tongue deviation A twelfth nerve palsy can

arise from a carotid artery dissection.

Mr Lennon gives a good description of his headache Th e gradual onset over

4 days makes a number of the more sinister causes less likely, specifi cally SAH In addition, one of the red fl ags is present: a new onset headache in someone older than

50 In such presentations, particularly given suggestive symptoms like possible jaw claudication, your priority is to exclude temporal arteritis.

Whilst you have begun to narrow your diagnosis, you still want to exclude sinister causes with your examination and investigations

What signs will you look for on clinical examination?

You start by characterizing Mr Lennon’s headache He tells you the pain is on the right side of his head and hasn’t ever moved It started 4 days ago, since when it has been getting worse He can only charac-terize it as intense He has tried over-the-counter analgesics with no benefi t, and when asked specifi -cally, says there is no change with position or time of day

He has had no changes in consciousness, nor seizures, that he is aware of When asked about other symptoms, he tells you he has found it hard to eat and open his mouth properly since yesterday because

of jaw pain He has not noticed any constitutional symptoms, and he hasn’t noticed any change in vision

He has never had anything like this before

How does this information help focus the diff erential diagnosis and your approach?

Core cases 5

1

• Horner’s syndrome Triad of partial ptosis, miosis (constricted pupil), and

anhydrosis (dry skin around the orbit) Results from interruption of the

ipsilateral sympathetic pathway In the context of our diff erential diagnosis,

Horner’s syndrome should raise suspicions of a carotid artery dissection (ask

about neck pain) or cavernous sinus lesion.

• Optic disc appearance on fundoscopy Look for papilloedema, indicating

raised intracranial pressure.

Other

• Reduced visual acuity Th is can suggest acute glaucoma or temporal arteritis

for example.

• Scalp tenderness Classically seen in temporal arteritis.

• Meningism Check whether the patient has a stiff neck or photophobia,

sug-gesting meningism due to infection or SAH

On examination, Mr Lennon is not obviously photophobic as he is sitting in a well-lit environment His

heart rate is 84 beats/min (bpm), his blood pressure is 134/81 mmHg, and his temperature 36.5°C

Examination of his cranial nerves reveals reduced visual acuity in his right eye but not his left, which

he previously hadn’t noticed Fundoscopy is normal The rest of his cranial nerves are intact but you do

notice that his right scalp is tender to light touch There are no limb signs and no neck stiff ness

Mr Lennon is an elderly man with a 4-day history of new-onset right-sided temporal headache,

possibly jaw claudication, a right-sided decrease in visual acuity, and a tender scalp

What is the most likely diagnosis? What is the pathology, and why is it an emergency?

Mr Lennon’s history and clinical features are highly suggestive of temporal

arteritis (aka giant cell arteritis, GCA) Th is is a disease of unknown aetiology that

typically appears in patients over 50 years of age It is characterized by the formation

of immune, infl ammatory granulomas in the tunica media of medium/large-sized

arteries Th e infl ammation (or thrombosis or spasm induced by it) can be suffi cient

to block the lumen of medium-sized arteries aff ected by this disease Infl

amma-tion of the mandibular branch of the external carotid artery causes jaw

claudica-tion Infl ammation of the superfi cial temporal branch of the external carotid artery

causes headache and scalp tenderness Infl ammation of the posterior ciliary arteries

causes visual disturbances, due to ischaemia to either the retina (blurring, visual

fi eld loss) or the optic motor muscles (double vision = diplopia).

Th e reason to worry about this presentation is that with visual loss in one eye the

other eye is at risk without prompt treatment Temporal arteritis with visual

distur-bance is therefore an ophthalmological emergency and patients should be referred

6 Headache

1

to the on-call ophthalmologist as soon as possible Unfortunately, visual loss prior to arrival at hospital is unlikely to be reversed regardless of treatment.

How will you proceed in light of your working diagnosis?

Having taken a full history and examined the patient, one should arrange only

fi rst-line investigations that are quick to do – such as blood tests to demonstrate

an elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)

that would be consistent with a systemic infl ammation such as temporal arteritis Management should then aim to reduce the immune-mediated infl ammation that

is causing the ischaemia in Mr Lennon’s arteries and the best way to do this is using

high-dose corticosteroids.

Once initial treatment is under way, one can arrange for more time-consuming investigations to help confi rm the diagnosis and rule out alternatives In this case,

a temporal artery biopsy should be arranged to help confi rm the diagnosis (it will

show granulomas in temporal arteritis) Note that the principal reason for urgent treatment of temporal arteritis is to protect the vision in the fellow, unaff ected eye, rather than aiming to restore vision to the aff ected eye.

Core cases 7

1

• Does the patient suff er from diff erent types of headache? If so, separate

histories will be needed for each as they may refl ect distinct syndromes Th us

patients with migraine are also vulnerable to medication overuse headaches

from the treatment for their migraine.

• Are there any predisposing (trigger) factors? Factors such as stress and

fatigue are known triggers for tension headaches and migraines Some

migraine suff erers point to certain foods as triggers (e.g cheese, caff eine), and

alcohol can trigger cluster headaches.

Tension-type headache

Migraine

Sinusitis

Medication overuse headache

Temporomandibular joint (TMJ) dysfunction syndrome (TMJ syndrome)

Trigeminal neuralgia

Cluster headache

Mr Lennon’s 40-year-old daughter has come to see her father You have explained that you think he has

temporal arteritis, an infl ammation of some of the blood vessels supplying his head Poor Miss Lennon

is worried that she may have the same problem as she also frequently gets headaches

You ask her to characterize the headache using SOCRATES She tells you the headaches only aff ect

the right side of her head They come on over half an hour, and make her feel nauseated and sensitive

to bright light and noise She only fi nds relief by hiding in a dark room and getting some sleep They last

hours, but less than a day, and are relatively infrequent, occurring three or four times a year since her

early twenties You also ask about the red fl ags, none of which are present

The lack of red fl ags makes a sinister cause of headache unlikely But there are several non-sinister

syndromes that cause headache These syndromes are not ‘benign’ because they cause signifi cant

morbidity (in the form of pain) However, they are unlikely to cause lasting damage or mortality in the

short term

What diff erent types of non-sinister headache are there?

Some of these are ‘primary headaches’ because the symptom (headache) is primary, i.e if the

head-aches were removed there would be no harmful pathology This is in contrast to ‘secondary headhead-aches’,

where the headache is only one of many possible symptoms that result from pathology such as head

trauma, intracranial lesion (e.g tumour), vascular lesion (e.g SAH), or infection The following are

sec-ondary headaches: sinusitis, medication overuse headache, and TMJ syndrome Sinusitis and TMJ

syn-drome cannot be diagnosed in the absence of additional symptoms A diagnosis of medication overuse

headache can only be made in patients using analgesic and/or migraine medication

In addition to the pain history (e.g SOCRATES), what questions should you ask to characterize

non-sinister headaches?

Causes of non-sinister headache include:

8 Headache

1

• How disabling are the headaches? Migraines render many suff erers

incapa-ble of performing even the activities of daily living for around a day Cluster headaches are severely painful and disabling but often occur at night, allow- ing daytime duties to continue Tension-type headaches usually allow normal activities to be continued.

• Does the patient get an ‘aura’ before the headache? Auras are usually

visu-al phenomena, visu-although focvisu-al neurologicvisu-al defi cits (e.g limb weakness) are sometimes present About a third of migraine suff erers report auras as a fea- ture of their migraines.

• Tension-type headaches Very common Often bifrontal pain Th ey are sodic, occurring with variable frequency Th e pain is described as pressure

epi-or tightness around the head like a tightening band Other than the ache there are no other features (e.g no photophobia) Th e headaches last no more than a few hours and are not severely disabling However, in rare cases they may occur almost daily, in which case they become disabling Stress and fatigue are well-known trigger factors.

head-• Migraine Common, although not as common as tension headaches, and twice

as common in women than men Migraines are stereotyped, i.e attacks

exhib-it the same pattern of symptoms and become recognizable to patients Th ey

are typically unilateral (migraine is a corruption of the Latin (he)mi-cranium)

Associated with an aura in about a third of suff erers (migraine with aura

or classical migraine, as opposed to migraine without aura or common

migraine) Th e pain is described as throbbing or pulsatile Th ere is sensitivity

to light, sound, and even smell, and nausea can also be a feature Migraines last between 4 and 72 hours, unless successfully treated Some patients suff er from

aura without migraine Such attacks are in the diff erential for TIAs

(particu-larly in older patients) and epilepsy.

• Sinusitis Patients usually report facial pain coming on over hours to days in

conjunction with coryzal symptoms Th e pain is tight, as in tension headaches, and is often exacerbated by movement Th e headaches last several days, with

a time course consistent with the infection Th e headaches are moderately severe but not disabling However, patients with chronic sinusitis may fi nd the headaches frequent enough to interfere with their daily activities.

fi ve-fold the incidence in men) Th is is seen particularly with migraine cations and analgesics Th e headaches experienced resemble either migraine

medi-or tension-type headaches Most patients will be taking very large quantities

of medication (on average 35 doses of six diff erent agents a week) It is often diffi cult for patients to accept that the over-treatment of headache is actually the cause of their ongoing headaches Treatment consists of withdrawal from analgesic use, which often results in a period of exacerbation before improve- ment occurs.

Going back to the list of diff erential diagnoses above, what are the key features of each diagnosis in the history?

Core cases 9

1

more prevalent in women As well as headache, patients get a dull ache in the

muscles of mastication that may radiate to the jaw and/or ear Patients also

often report hearing a ‘click’ or grinding noise when they move their jaw.

• Trigeminal neuralgia A rare condition, occurring more often in women,

with a typical age of onset around 60–70 years Patients complain of

unilat-eral facial pain involving one or more of the divisions of the trigeminal nerve

Th e pain lasts only seconds, and can be triggered by eating, laughing, talking

or touching the aff ected area Although attacks last seconds, there may be

several or even hundreds a day and patients can develop a longer-lasting

back-ground pain Patients often avoid known triggers like shaving Interestingly,

attacks rarely occur during sleep, unlike migraine or cluster headaches.

• Cluster headache Predominantly aff ects men Th e headaches occur in

‘clus-ters’ for about 6–12 weeks every 1–2 years, hence the name Attacks tend to

occur at exactly the same time every day or night, like an alarm clock going

off Th e pain is focused over one eye Th e pain is intense and causes the patient

to wake up and can be so severe that suicide is contemplated, until the pain

diminishes, around 20–30 minutes later Th ey will probably have a red, watery

eye, rhinorrhoea, and Horner’s syndrome, suggested by a history of ptosis

Th ese headaches are very disabling.

You ask the additional questions listed above Miss Lennon tells you she sometimes gets ‘normal’

head-aches, which respond to paracetamol, but it is these other headaches that she worries about When you

ask about any ‘aura’ or visual disturbance, she tells you they are often preceded by seeing a small black

spot with bright, zig-zagging lines When she has a headache, she has to stay in bed all day until it goes,

but will be fi t and well the day after

What is the most likely diagnosis, given Miss Lennon’s history?

Should you examine Miss Lennon and, if so, what would you look for? Should you order any

investiga-tions and, if so, which?

It may be tempting to dismiss Miss Lennon’s headaches as tension headaches,

which almost everyone experiences at some point However, Miss Lennon describes

a unilateral headache that makes her feel sick, photophobic, phonophobic, and

is preceded by a visual phenomena (zig-zag lines) It is therefore very likely that

Miss Lennon suff ers from migraine with aura.

Migraine aff ects about 15% of the population, but many do not seek help as they

think there is no treatment Th is is not true, as acute abortive treatment

includ-ing triptans (5HT1-agonists such as sumatriptan), analgesics (aspirin, paracetamol),

and anti-emetics (metoclopramide) have been shown to be highly eff ective if the

patient takes them as soon as they feel a migraine coming on – something that most

migraine suff erers recognize easily Preventative treatments are only useful with

high frequencies of attacks (e.g fortnightly) and will usually only reduce migraine

frequency by 50%.

10 Headache

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All of the non-sinister causes of headache are diagnosed on history However, you should conduct a physical examination, both to provide the patient with reassur- ance and to look for:

• Blood pressure, to exclude malignant hypertension.

• Head and neck examination for muscle tenderness, stiff ness, or limited

move-ment – which can occasionally mimic tension-type headaches If present, such

fi ndings may need treatment in order to relieve the headache.

• Focal neurological signs Th e presence of focal neurological signs in body with headache should alert you to intracranial pathology

some-• Fundoscopy, to exclude raised intracranial pressure.

Investigations are only ever indicated where warranted by the history and nation and should not be ordered routinely.

Short cases

Mrs Harrison is a 42-year-old who presents to accident and emergency (A&E) complaining of a severe

headache and nausea She has a history of migraine attacks but this time she says it is diff erent – it

came on suddenly after dinner, without warning, and felt as if someone had punched her in the back

of the head Her husband, annoyed at having to drive her to the hospital in the middle of the night,

cynically thinks she is just having ‘a bad migraine’

What is the likely diagnosis? What key investigation should be requested?

Patients know their disease better than any doctor, so if a patient tells you that

something doesn’t feel like what they normally have (e.g migraines in this case),

take them seriously You should take a full history and examine the lady for key signs

as discussed in the main case (e.g neck stiff ness suggesting meningism) But the

history given is classic for an SAH, so you should arrange an urgent computed

tom-ography (CT) head scan, looking for blood in the CSF (this appears bright on CT,

for example in the Sylvian fi ssures) An LP looking for xanthochromia (yellow CSF

due to bilirubin content) must be performed to exclude SAH if the CT is negative

Note that CT is only useful as an aid to diagnosis of SAH in the fi rst days following a

bleed – by approximately day 7 the scan will have ~50% sensitivity (i.e you might as

well fl ip a coin) LP should be delayed for 12 hours after the onset of the headache as

false negative results can occur before that time It remains reliable for up to 12 days

(12 hour to 12 day rule).

If the CT confi rms an SAH, Mrs Harrison will need urgent referral to a

neurosurgi-cal unit Patients are initially managed with nimodipine (a neurosurgi-calcium-channel blocker

that reduces spasm of the ruptured cerebral artery, thus preventing ischaemia, i.e

a stroke) and bed rest

If she survives and her symptoms improve, she should receive cerebral

angiogra-phy to fi nd the source of a bleed – usually a ruptured aneurysm Th e

neuroradiolo-gist will usually be able to insert a platinum coil to cause the aneurysm to clot, scar,

and heal Coiling has been shown by the ISAT Study1 to have fewer complications

than surgically clipping the aneurysm via an open craniotomy Th e family should be

made aware that SAH carries a high risk of mortality and morbidity: 50% of patients

die before arriving at hospital; a further 17% die in hospital; another 17% survive but

with lasting neurological defi cits; and only 17% survive without any sequelae.

1 Molyneux AJ, Kerr RS, Yu LM, Clarke M, Sneade M, Yarnold JA, Sandercock P (2005)

Interna-tional subarachnoid aneurysm trial (ISAT) of neurosurgical clipping versus endovascular coiling in 2143

patients with ruptured intracranial aneurysms: a randomised comparison of eff ects on survival,

depend-ency, seizures, rebleeding, subgroups, and aneurysm occlusion Lancet, 366: 809–817.

12 Headache

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TIAs and strokes are caused by areas of the brain ceasing to function due to a lack

of blood (because of an embolism or haemorrhage) and are therefore characterized

by ‘negative’, loss of function, symptoms and signs (loss of vision, numbness, loss

of power in muscles) In contrast, epilepsy is caused by over-activation of areas of

the brain and thus produces ‘positive’, gain of function, symptoms and signs (fl

ash-ing lights, muscle convulsions, odd sensations in the skin) Migraine can produce both negative and positive symptoms Th is gentleman describes clear gain of func- tion symptoms (shimmering light, noises) that are more suggestive of epilepsy or migraine.

It could be epilepsy, but seizures tend to be followed by a post-ictal phase where the patient is exhausted and sometimes confused It is therefore more likely to be a

case of migraine aura without headache A characteristic feature is a slow march of

symptoms (e.g visual disturbance aff ecting more and more of the visual fi eld) then resolution in a similar fashion Migraine without aura is an odd condition where patients experience the aura signs of typical migraine patients (e.g shimmering lights with ziz-zagging edges, noises) but without the headache One can reassure the patient that his symptoms are almost certainly not ‘mini-strokes’ but more likely

due to a type of migraine without headaches Th e patient may wish to have a trial of antimigraine medication as soon as one of these attacks starts (e.g sumatriptan) or prophylactic medication to prevent it occurring (e.g propanolol, pizitofen) and see

if they help.

Mr McCartney is a 32-year-old salesman who is worried that he might be having repeated ‘mini-strokes’ (TIAs), like his father He says that every couple of months he suff ers from an attack where he sees a shimmering light in the corner of his eyes and gets a ringing in his ears This usually occurs towards the end of the day, lasting half an hour He is fully conscious throughout and never feels dazed or confused afterwards

Could this gentleman be suff ering from TIAs like his father?

It is possible that this young man simply has a viral rhinosinusitis, but the green

mucus and the highly localized pain above his eyes suggest he may have developed

an infection of his frontal sinuses.

Sinusitis usually aff ects the maxillary sinus and resolves spontaneously,

occasion-ally needing a helping hand from antibiotics (e.g amoxicillin) if it fails to resolve

However, frontal sinusitis can be particularly dangerous because it is possible

for the bacteria to erode backwards into the brain, causing meningitis or a brain

abscess For this reason, a suspected case of frontal sinusitis should be taken

seri-ously and referred to ear, nose, and throat (ENT) specialists who can arrange a CT

head scan to check if either frontal sinus is aff ected If it is, he will need antibiotics

and draining of the frontal sinuses (antral lavage) It is possible that his deviated

nasal septum is predisposing him to episodes of sinusitis and, if so, he could benefi t

from a re-arrangement of his nasal septum (septoplasty).

Ringo is a 16-year-old who presents with a runny nose and headache He has been blowing out green

mucus from his nose for a few days but has come to see you because the headache, which is located

above his eyes, is now very bad His nasal septum is slightly deviated and his forehead is indeed tender

to gentle tapping

Can we send this patient home on analgesia and rest?

14 Headache

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Th e appearance of the optic disc (poorly defi ned margins) is suggestive of

papill-oedema, potentially due to raised intracranial pressure Th is would be consistent with headaches which are worse in the morning One cause of raised intracranial pressure is brain tumour, and in children most brain tumours are found in the pos- terior fossa that would explain the occipital headache In addition, the most com- mon type of tumour in children is a medulloblastoma in the cerebellum, which may explain her clumsiness Th us you must rule out a CNS tumour as the problem in this child An urgent head magnetic resonance imaging (MRI) scan should there-

fore be requested.

If a tumour is found, the neurosurgeon will use dexamethasone to reduce the brain infl ammation (improving the headache) and discuss the surgical options to remove the tumour with the family.

A 10-year-old girl is referred to the hospital by her GP for persistent headache in the occipital area that

is worse in the morning The parents have also noticed that she has become clumsy over the last few months As part of your cranial nerve examination you perform fundoscopy, where you see the result shown in Fig 1.1

Figure 1.1 Fundoscopic view of the patient’s retina Both retinas had a similar appearance

Reproduced from Fig 1, p.449, in Oxford Handbook of Clinical Specialities, 7th edn, by Collier, Longmore, and Brinsden (2006)

By permission of Oxford University Press.

What is your next step?

The main causes of SAH are:

• Rupture of an arterial aneurysm, usually a ‘berry aneurysm’ at the junction between arteries of the

circle of Willis (~45%)

• Trauma (~45%)

• Arteriovenous malformations, rupture of haemangiomas, rupture of cerebral vein around the

brain-stem (~10%)

The list of possible intracranial tumours is very long but the most common are:

• Secondary brain tumours (metastatic) These are the most common type of brain tumours in adults,

accounting for ~90% of all intracranial tumours The fi ve most common sources of primary cancer

metastasizing to the brain are lung, kidney, breast, melanoma, and colon

• Primary brain tumours These can be divided into axial (within the brain parenchyma) and extra-axial:

Axial

− or neuroepithelial tumours (~50%) These are tumours of the brain matter itself They include

(the latter three are more common in children):

Astrocytomas (glioblastoma multiforme is a grade 4 astrocytoma)

Meningioma (~15%) A slow-growing tumour of the meninges that compresses the brain Often

associated with neurofi bromatosis type II (inherited predisposition to schwannomas and

menin-giomas), these tumours can usually be surgically removed with good prognosis

Vestibular schwannoma Previously and incorrectly called an ‘acoustic neuroma’, this type of tumour

is relatively common in young adults It may compress cranial nerve VIII (hearing loss) and VII

(facial palsy)

Pituitary adenomas, prolactinomas, and craniopharyngiomas

Other: choroid plexus papillomas, haemangiomas, pineal gland tumours, etc

Viva questions

What are the main causes of SAH?

What is your diff erential diagnosis for intracranial tumours?